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      Clinical manifestations of IgA nephropathy combined with thin glomerular basement membrane nephropathy in children

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          Abstract

          Background

          Immunoglobulin A nephropathy (IgAN) and thin glomerular basement membrane nephropathy (TBMN) are the most common causes of persistent hematuria during childhood. The objective of this study is to determine the difference in clinical features and laboratory findings between pediatric patients with IgA deposited TBMN and IgAN alone.

          Methods

          Between January 2000 and March 2009, 95 children were diagnosed with IgAN by renal biopsy. Clinical features and laboratory findings of patients with isolated IgAN and with IgAN plus TBMN were compared; the children diagnosed with IgAN were compared to 127 children who had been diagnosed with TBMN alone during the same period.

          Results

          There were 71 (74.7%) of a total 95 patients that were diagnosed with isolated IgAN (Group1); in 24 (25.3%) of the 95 patients IgAN was combined with TBMN (Group 2). There was marked difference in the gender distribution between Group 2 and isolated TBMN patients. The degree of proteinuria and pathologic severity was higher in Group 1 compared with Group 2. Gross hematuria was present in both groups. There were no distinguishing features in the other laboratory parameters.

          Conclusion

          Patients with both IgAN and TBMN seem to have similar clinical features to patients with isolated IgAN; however, the latter tend to have better pathologic and laboratory findings, compared to the patients with IgAN alone.

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          Histologic subclassification of IgA nephropathy: a clinicopathologic study of 244 cases.

          Christine M. Haas (1997)
          IgA nephropathy (IgAN) may present with a wide variety of histologic patterns on renal biopsy, ranging from a minimal lesion to a diffuse proliferative glomerulonephritis (GN). The histologic features of 244 cases of IgAN (not including Schönlein-Hanoch nephritis) diagnosed between 1980 and 1994 were reviewed, and each case was subclassified using the following, relatively simple histologic classification scheme: subclass I (39 cases): minimal or no mesangial hypercellularity, without glomerular sclerosis; subclass II (18 cases): focal and segmental glomerular sclerosis without active cellular proliferation; subclass III (110 cases): focal proliferative GN; and subclass IV (42 cases): diffuse proliferative GN; and subclass V (35 cases): any biopsy showing > or = 40% globally sclerotic glomeruli and/or > or = 40% estimated cortical tubular atrophy or loss. Subsequent analysis of renal survival in 109 patients who underwent biopsy before or during 1992 for whom such data were available showed a strong, statistically significant correlation between histologic subclass and renal survival, with an order I, II (greatest survival) > III > IV, V. Crescents were a significant negative prognostic indicator for renal survival in subclass III (but not in subclass IV), and interstitial expansion was a negative prognostic indicator in subclasses III and IV, although the statistical significance of these were not maintained after controlling for serum creatinine at the time of biopsy. The presence of peripheral glomerular capillary deposits ultrastructurally had no prognostic significance. With respect to clinical presentation, hypertension (systolic blood pressure > or = 130 mm Hg and diastolic blood pressure > or = 90 mm Hg) and proteinuria of > or = 2.0 g/24 hr were significant negative prognostic indicators for renal survival, even when controlling for serum creatinine at the time of renal biopsy. The presence of gross hematuria correlated significantly with increased renal survival by univariate analysis, but not when controlling for serum creatinine at the time of renal biopsy. The findings of this study confirm the wide variety of clinical and histopathologic presentations of IgAN, and indicate the utility of the proposed histologic classification schema in assessing a patient's likelihood of ultimately developing end-stage renal disease.
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            IgA nephropathy: long-term prognosis for pediatric patients.

            Robert J. Wyatt, Stephen Kritchevsky, Susan Woodford … (1995)
            The determination of the ultimate prognosis for patients with IgA nephropathy diagnosed in childhood requires long-term follow-up of identified patients. The purpose of this study was to obtain such follow-up for patients from two centers where the disease has been diagnosed for more than 20 years. Clinical data at the apparent onset of symptoms and renal histologic data were obtained for 103 patients in whom IgA nephropathy was diagnosed before age 18 years. Clinical status at last follow-up was obtained from office records or from direct contact with the patient. Predicted kidney survival was determined by the Kaplan-Meier method. Follow-up of more than 10 years from the time of biopsy was available for 40 of the patients. Fourteen of the patients have progressed to end-stage renal disease; three others have progressive chronic renal insufficiency as defined by an estimated creatinine clearance of less than 50 ml/min per 1.73 m2. Severity of the renal histologic findings and the degree of proteinuria at the time of biopsy were associated with poor outcome. For all patients, predicted kidney survival from the time of apparent onset was 94% at 5 years, 87% at 10 years, 82% at 15 years, and 70% at 20 years. Age at clinical onset and gender were not associated with poor outcome, but black race and severity of renal histologic findings were. With follow-up into adulthood, the outcome for pediatric patients with IgA nephropathy appears to be as serious as that reported in adult patients. Follow-up of a pediatric patient with persistent clinical findings should be maintained after the patient's care is transferred to a physician caring for adults.
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              Hematuria and proteinuria in a mass school urine screening test.

              Hyo Kyun Chung, Jun Yoo, Ja-Won Koo … (2005)
              A total of 1,044 school children identified with hematuria and/or proteinuria during a mass school urine screening test were referred to pediatric nephrologists at 13 hospitals in Korea. These children had isolated hematuria (IH) (60.1%), isolated proteinuria (IP) (26.4%: transient, 19.6%; orthostatic, 4.9%; persistent, 1.9%) or combined hematuria and proteinuria (CHP) (13.5%). The patient's history, physical examination, laboratory tests, kidney ultrasound and Doppler ultrasonography were obtained. Renal biopsies were performed on 113 children who showed severe proteinuria, hypertension, abnormal renal function, family history of chronic renal disease, systemic diseases or persistent hematuria and/or proteinuria for more than 12 months. IgA nephropathy (IgAN), thin basement membrane nephropathy (TBMN), membranoproliferative glomerulonephritis (MPGN), focal segmental glomerulosclerosis (FSGS), other GN, Alport syndrome and lupus nephritis were detected. IgAN and TBMN were the most common causes in the CHP group and IH group, respectively. Abnormal findings on the renal ultrasound with or without Doppler ultrasonography were noted in 147 cases (suspected nutcracker phenomenon, 65; increased parenchymal echogenicity, 40; hydronephrosis, 15). This study showed that the use of a mass school urine screening program can detect chronic renal disease in its early stage and recommends that more attention should be paid to identifying those children with CHP and massive proteinuria. A school urine screening program can detect chronic renal disease in its early stage. When mass screening is used, the initial aggressive diagnostic procedures such as renal biopsy are not needed. In addition, a regular follow-up for those children with IH and IP is certainly warranted.
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                Author and article information

                Contributors
                Min Hyun Cho
                Journal
                Journal ID (nlm-ta): Kidney Res Clin Pract
                Journal ID (iso-abbrev): Kidney Res Clin Pract
                Title: Kidney Research and Clinical Practice
                Publisher: Elsevier
                ISSN (Print): 2211-9132
                ISSN (Electronic): 2211-9140
                Publication date PMC-release: 04 September 2013
                Publication date (Print): September 2013
                Publication date (Electronic): 04 September 2013
                Volume: 32
                Issue: 3
                Pages: 111-114
                Affiliations
                [1 ]Department of Pediatrics, Kyungpook National University, School of Medicine, Daegu, Korea
                [2 ]Department of Pathology, Kyungpook National University, School of Medicine, Daegu, Korea
                Author notes
                [* ]Corresponding author. Department of Pediatrics, Kyungpook National University, School of Medicine, 807 Dongdeok-ro, Jung-gu, Daegu 700-721, Korea. chomh@ 123456knu.ac.kr
                Article
                Publisher ID: S2211-9132(13)00057-0
                DOI: 10.1016/j.krcp.2013.07.001
                PMC ID: 4714097
                PubMed ID: 26877925
                SO-VID: 53fee66d-34e4-4c4f-b2ee-39eebeb7f7c5
                Copyright © © 2013. The Korean Society of Nephrology. Published by Elsevier.
                License:

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                Date received : 2 April 2013
                Date revision received : 4 June 2013
                Date accepted : 5 July 2013
                Categories
                Subject: Original Article

                Keywords: children,immunoglobulin a nephropathy,thin glomerular basement membrane nephropathy
                Data availability:
                Keywords: children, immunoglobulin a nephropathy, thin glomerular basement membrane nephropathy

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